Abstract
There is an evolving understanding that autoimmune pancreatitis (AIP) is an immunoglobulin (Ig) G4 systemic disease. It can manifest as primarily a pancreatic disorder or in association with other disorders of presumed autoimmune cause. Classic clinical characteristics include obstructive jaundice, abdominal pain, and acute pancreatitis. Thus, AIP can be difficult to distinguish from pancreatic malignancy. However, AIP may respond to therapy with corticosteroids, and has a strong association with other immune mediated diseases. Although primarily a pathologic diagnosis, attempts have been made to reliably diagnose AIP clinically. AIP can be classified as either type 1 or type 2.
Keywords:
Autoimmune pancreatitis; Corticosteroids; Idiopathic duct-centric pancreatitis; IgG4; International Consensus Diagnostic Criteria; Lymphoplasmacytic sclerosing pancreatitis.
Copyright © 2016 Elsevier Inc. All rights reserved.
MeSH terms
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Adrenal Cortex Hormones / therapeutic use*
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Autoimmune Diseases / diagnostic imaging
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Autoimmune Diseases / drug therapy*
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Autoimmune Diseases / immunology
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Autoimmune Diseases / pathology
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Azathioprine / therapeutic use
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Biopsy
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Cholangiopancreatography, Endoscopic Retrograde
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Cyclophosphamide / therapeutic use
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Cyclosporine / therapeutic use
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Disease Management
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Endosonography
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Humans
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Immunoglobulin G / immunology
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Immunologic Factors / therapeutic use*
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Immunosuppressive Agents / therapeutic use*
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Mercaptopurine / therapeutic use
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Pancreas / diagnostic imaging
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Pancreas / pathology*
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Pancreatitis / diagnostic imaging
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Pancreatitis / drug therapy*
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Pancreatitis / immunology
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Pancreatitis / pathology
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Rituximab / therapeutic use
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Tomography, X-Ray Computed
Substances
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Adrenal Cortex Hormones
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Immunoglobulin G
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Immunologic Factors
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Immunosuppressive Agents
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Rituximab
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Cyclosporine
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Cyclophosphamide
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Mercaptopurine
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Azathioprine